Gangguan pendengaran dan gangguan jalan napas bagian atas pada Mukopolisakaridosis tipe II

Oto Rhino Laryngologica Indonesiana

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Title Gangguan pendengaran dan gangguan jalan napas bagian atas pada Mukopolisakaridosis tipe II
 
Creator Airlangga, Tri Juda
 
Subject mukopolisakaridosis tipe II, GAG, penyakit terkait kromosom X, gangguan pendengaran, OSA
 
Description Latar belakang: Penyakit Mukopolisakaridosis (MPS) tipe II atau sindrom Hunter adalah gangguan terkait dengan kromosom X resesif yang disebabkan defisiensi enzim iduronate-2-sulfatase sehingga memicu penimbunan heparan sulfat dan dermatan sulfat. Gangguan pada pendengaran dan pernapasan bagian atas merupakan masalah umum yang terjadi pada penyakit ini. Tujuan: Untuk memberikan informasi mengenai gangguan pendengaran dan gangguan pernapasan bagian atas pada penyakit MPS tipe II. Kasus: Tiga pasien terdiagnosis MPS tipe II dengan gangguan pendengaran dan hipertrofi adenoid. Metode: Didapatkan 4 jurnal yang relevan setelah dilakukan penelusuran literatur dan penapisan dengan kriteria inklusi dan eksklusi. Hasil: Empat jurnal yang terdiri dari 3 laporan kasus dan 1 artikel penelitian menggambarkan gangguan pendengaran dan gangguan jalan napas bagian atas pada pasien dengan mukopolisakaridosis tipe II. Kesimpulan: Pendekatan multidisipliner diperlukan untuk mendiagnosis serta mengobati pasien MPS tipe II secara dini, agar dapat menunjang perkembangan individu dan sosial serta meningkatkan kualitas hidup pasien. Background: Mucopolysaccharidoses (MPS) type II is X-linked recessive disorder caused by iduronate-2-sulfatase enzyme deficiency and leads to the accumulation of heparan sulfate and dermatan sulfate. Hearing loss and upper respiratory track problems are commonlyoccur in this disorder. Purpose:To provide information about hearing loss and upper airway problems in MPS type II disorder. Cases: Three patients had been diagnosed as mucopolysaccharidoses type II with hearing loss and adenoid hypertrophy.Methods: Four relevant journals were obtained after literature searching and screening was performed using inclusion and exclusion criteria. Results: Out of 4 journals, 3 case reports and 1 research article were describing hearing loss and upper respiratory problems in patients with MPS type II. Conclusion:Multidisciplinary approach is needed for making early diagnosis and determining the treatment of mucopolysaccharidoses type II patients, in order to support individual and social development and to improve the patients’quality of life.
 
Publisher PERHATI-KL
 
Contributor
 
Date 2019-01-30
 
Type info:eu-repo/semantics/article
info:eu-repo/semantics/publishedVersion

 
Format application/pdf
 
Identifier http://orli.or.id/index.php/orli/article/view/244
10.32637/orli.v48i2.244
 
Source Oto Rhino Laryngologica Indonesiana; Vol 48, No 2 (2018): Volume 48, No. 2 July - December 2018; 173-179
2598-3970
0216-3667
10.32637/orli.v48i2
 
Language eng
 
Relation http://orli.or.id/index.php/orli/article/view/244/186
 
Rights Copyright (c) 2019 Oto Rhino Laryngologica Indonesiana
 

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